Giant trichoblastoma of the scalp

Trichoblastoma is a rare and benign adnexial tumor with characteristic histological features. It occurs on any hair folliclebearing location, and usually presents as a solitary lesion most often less than 2 cm in size. Giant trichoblastoma has been rarely reported in the literature. To report a new case of giant trichoblastoma, misleading for malignancy. A 57-year-old woman presented with a 5 cm-solitary asymptomatic nodular lesion of the scalp, of 28 years. It had been previously excised with recurrence and progressive regrowth. On examination, it was a dome-shaped, erythematous, firm, papillomatous, non infiltrated nodule. Full body work up revealed no metastases. Cutaneous biopsy concluded to trichoblastoma but failed to eliminate malignancy. After excision with secondary skin graft, histological examination confirmed the benignity with clear margins. There was no evidence of recurrence after a 5 year-follow-up period. This case illustrates a rare clinical variant of trichoblastoma with an unusual important size. This can be misleading for malignancy, but the slowly progressive course of the tumour in our patient, together with histological benignity led to the correct diagnosis. This tumour is considered as a distinct entity by some authors

[Hydroxyurea induced-Leg ulcer in polycythemia vera]

Hydroxyurea [HU] is an antineoplastic drug commonly used to treat chronic myeloproliferative disorders. Dermatological side effects are frequent and usually benign. Leg ulceration following HU therapy is less common. To describe epidemioclinical and therapeutic features of leg ulcers induced by HU. A 70-year-old woman is treated with hydroxyurea for polycythemia vera. One year later; she presented with a malleolar painful ulcer, initially healed without discontinuation of the treatment, but has been recurred 2 months later, becoming multiple and bilateral. HU has been discontinued and ulcers were completely cured. Leg ulcers induced by hydroxyurea are rare. Pathogenesis of HU-induced ulcers remains unknown and is multi factorial. Discontinuation of treatment is still the option of choice for complete recovery

[Rosacea: 244 Tunisian cases]

Rosacea is a common, chronic facial dermatosis of uncertain etiology, several well-defined types and variable progression. There is a paucity of epidemiologic studies from North African countries especially in Tunisia. To determine epidemiological, clinical, histological features, treatment and outcome of rosacea in a Tunisian study. A tretrospective study of all rosacea cases diagnosed in the outpatient Dermatology Department of Charles Nicolle hospital of Tunis was conducted between 1990, January and 2003, May. Our study included 244 patients. The diagnosis of rosacea was made on symptoms and clinical features in 185 cases and on histological findings in 59 patients. The hospital prevalence of rosacea was 0.2%. The sex ratio M/F was 0,4. Patient's Mean age was of 49 years. Triggered factors mentioned included most often: sun exposure [64%] and thermal stimuli [25%]. Mean duration between the onset of symptoms and the first consultation was about 20 months. The rosacea subtypes were: erythematotelangiectatic type [12%], papulopustular rosacea [69%], and rhinophyma [3.7%]. Granulomatous rasacea was diagnosed in 8 patients and steroid rosacea in 28 cases. Ocular rosacea was observed in 41 cases. Treatment modalities included: topical treatment [1.6%], systemic treatment [85%], systemic and topical treatment [5%] and surgical procedures [1.2%]. Rosacea seems to be a frequent dermatosis in Tunisia where most of the population is phenotype IV-V. It affects mostly middle-aged women. Most of our patients present with papulopustular rosacea [69%]. More epidemiological and clinical studies in North African countries should be conducted to emphasize these results

[Chronic lupus erythematosus: 104 Tunisian cases]

Chronic lupus erythematosus is a cutaneous form of lupus eryhematosus, usually involving photoexposed areas. The aim of our study is to evaluate the epidemio-clinical trends, therapeutic features and outcome of patients with chronic lupus erythematosus, through a Tunisian hospital series. We have tried to compare our results with those of other African and western series. It is a retrospective study, concerning all patients with chronic lupus erythematosus, attending the Dermatology department of Charles Nicolle Hospital over an 11 years period. The patients' age, sex, clinical features, explorations results, treatment and evolution were recorded. A total of 104 patients were included. Chronic lupus erythematosus represented 0,1% of all the dermatitis seen over 11 years. Chronic lupus erythematosus affects young women with a ratio F/M of 1,97 and an average age of 42 years. The discoid form was the most frequent clinical shape, observed in 73% of cases [76 patients]. The face was the most frequent localization of the lesions [91%]. Five patients [4.8%] presented a generalized chronic lupus erythematosus. The direct immunofluorescence in stick skin was positive in 54% of cases. Treatment included sun avoidance and oral antimalarials drugs in the majority of cases [95%]. The progression from chronic lupus erythematosus to systemic lupus erythemetosus was observed in two cases [2%]. Chronic lupus erythematosus is a relatively are disorder in Tunisia, compared to other African countries, where prevalence is 7 to 10 times more frequent. As shown in our study, the most common clinical feature is the discoid form. The diagnosis can be confirmed by histopathological and direct immunofluorescence examination. Progression of chronic lupus erythematosus to systemic lupus erythematosus is possible, as observed in tow of our patients. Thus, patients with chronic lupus erythematosus should be continually followed up

Cutaneous, pulmonary and sinusal aspergillosis in a diabetic patient

Cutaneous aspergillosis is rarely reported in diabetic patients. The objective of our study is to report a case of lethal disseminated aspergillosis revealed by multiples skin necroses with pulmonary and sinusal involvement in a diabetic patient. A 60-year-old diabetic woman. presented wins one month-rapidly-extensive, 1 to 10 cm skin necroses of the trunk limbs and eyelids. Few days after her admission. she developed dyspnoea. Chest X-ray showed an interstitial and alveolar syndrome with multiple excavated anfractuous-edged-opacities. Facial CT scan showed a right orbital cellulitis with Pansinusitis. The methamine-silver stains on a cutaneous biopsy showed filamentous septate fungal hyphae with branches at right angles. The immunofluorescence with an anti-aspergillus serum was positive. The diagnosis of secondary disseminated aspergillosis to a pulmonary focus with cutaneous, sinusal, and upper airway's dissemination was made. The patient died despite an intravenous amphotericin B therapy. This report emphasizes the importance of evoking and seeking for a mycosis in every skin necrotic and ulcerative lesions occurring in an immunocompromised patient. The prognosis den on the diagnosis and treatment institution delay

[Side effects of meglumine antimoniate in cutaneous leishmaniasis: 15 cases]

Leishmaniasis is wide spread parasitic disease considered to be endemic in 88 countries in both old and new world. The standard treatment remains Meglumine antimoniate. We study the side effects of systemic meglumine antimoniate in cutaneous leishmaniasis. We conduct a retrospective study covering 3-year period [2002- 2005]. All medical reports of cutaneous leishmaniasis treated by systemic Meglumine antimoniate are reviewed. The study comprise 63 patients all treated by systemic meglumine antimoniate at the dose of 60mg/kg/day for 10-15 days. Side effects were noted in 15 cases [12 females and 3 males] .The subject's age range from 11 to 78 years. Stibio-intolerance [fever, rash, arthralgia, abdominal pain] was observed in 12 cases and stibio-toxicity in 3 cases: precordialgies 1 case, hyperamylasemia and increase liver enzyme: 1 case, pancytopenia, renal and hepatic failure leading to death: 1 case, skin eruption: 7 cases, pruritis and erythema in the site of injection: 5 cases, urticaria: 1 case. Meglumine antimoniate was stopped in 13 cases Meglumine antimoniate is the generally recommended treatment of cutaneous leishmaniasis. In spite of the rarity of Glucantime's side effects, we recommend a careful survey especially in older patients

[Chronic prurigo revealing an angio-immunoblastic T cell lymphoma]

Cutaneous manifestations in angio-immunoblastic T cell lymphoma [AITL] can be seen in almost 50% of patients. They are especially represented by maculo-papular pseudotoxic exanthema. The other manifestations, like nodular prurigo are rarely observed. We report a case of AITL diagnosed after an etiologic screening for chronic prurigo. The objective of our work is to stress on the possibility to diagnose a hemopathy in etiologic screening of chronic pruritus. A 45-year-old patient presented a chronic pruritus of 18 months associated with general manifestations [fever, night sweating and weight loss]. Physical examination showed diffuse adenomegalies. On histology, the lymph nodes were composed of polymorphous lymphoid proliferation made of middle to large sized cells with clusters of epithelioid cells and post-capillary veinules hyperplacia. Immunohistochemical study showed T cell phenotype [CD3+]. Large cells were CD30+. Latent Protein of EBV virus was not expressed. Molecular biological analysis of a lymph node showed a T cell clonal proliferation. Cutaneous biopsy showed a little dermic perivascular lymphocytic inflammatory infiltrate. The diagnosis of angio-immunoblastic T cell lymphoma was made. The abdomino-pelvian CT scanner showed multiple inter-aortico-cave lymph nodes and a splenomegaly. A polychemotherapy was initiated. In front of chronic prurigo with general manifestations, a careful etiologic screening should be done to detect internal disorders especially malignant hemopathies

[Localized scleroderma: a retrospective study. about 92 cases]

Sclerodermas are rare affections which can be located or generalized Localized form is the most frequent. The purpose of this study was to describe epidemiologic, clinics, biological, immunological, therapeutic, evolutionary characteristics of the localized scleroderma through a personal series and the data of the literature We have performed a retrospective study on all patients followed in the department of dermatology of the Hospital Charles Nicole during 14]ears period. Our study was about 92 cases of localized scleroderma [73 were females and 19 males]. The mean age was 35 years [between 2 and 72 years]. The majority of localised sclerodermas [66 2%of the cases] appeared before 40 years with a maximum of frequency between 10 and 30 years [41,6%]. Only 11.9%of the cases were observed before 10 years. They were 51 cases [55%] of morphea, 35 cases [38%] of scleroderma in bands including 32 linear scleroderma and 3 scleroderma en coup de sabre, 5 cases [5,5%] of generalized morphea and 1 case [0,15%] of deep morphea. Average therapeutic was specified among 63 patients [87%], and the evolution could be appreciated among 45 patients. The epidemiologic data observed in our series are comparable with those reported in the literature. Therapeutic difficulties and risks of functional after-effects, particular in scleroderma in bands, remain the principal concern for all the authors

[Orf of the hand]

The orf is a z0000tic infection which can be transmitted to humans. The aim of our report is to describe this often misdiagnosed viral infection in a man with an orf of the hand appearing after the feast of the sacrifice. A 40-year-old man with no past medical history, presented with a nodular lesion on the fifth right finger evolving for 3 weeks which had appeared 2 weeks after the feast of the sacrifice. Histology showed a vacuolar degeneration of keratinocytes probably of viral origin. Both histological aspect and the contact with sheep, led to the diagnosis of hand orf. Three weeks later the lesion had spontaneously resolved. Orf is an infectious mucocutaneous disease due to a parapoxvirus. It is more frequent in professionally exposed persons [veterinary, butchers]. But, in our country, the frequency of the orf increases after the feast of the sacrifice and remains undiagnosed because patients do not consult. The diagnosis may be confirmed by electron microscopy, conventional histopathology or by isolation of the virus by PCR. Information on the benign character of the disease and reassurance of the infected patient are very important because lesion usually resolves spontaneously

[Hypohidrotic ectodermal dysplasia: ten cases]

Ectodermal dysplasias are rare hereditary diseases characterised by congenital absence of ectodermally derived structures and classified according to four symptoms: trichodysplasia, hypodontia, onychodysplasia and hypohidrosis. The objective of our study is to precise the epidemioclinical characteristics, the diagnostic tools, the evolution and the treatments of this rare disease through a 10-case series of hypohidrotic ectodermal dysplasia [HED]. The present report is a retrospective study of all cases of an/hypohidrotic ectodermal dysplasia collected from 1977 to 2006. We have specified age, sex, parental consanguinity, similar familial cases, clinical and histological features, dental, oto-rhino-laryngologic, ophthalmologic and respiratory examinations. Ten cases of HED were collected [average age: 14 years, sex ratio 9/1]. The mean duration diagnostic period was of 14 years. Parental consanguinity was registered in 3 cases but only one patient had similar familial cases. All patients had facial dysmorphy, hypotrichosis and hypo/anodontia [respectively 8/10 and 2/10]. All patients had clinically and histologically documented hypoplastic [6/10] or aplastic sweat glands [4/10]. Extra-cutaneons manifestations were noted in 8 patients [recurrent rhinitis 6/10, recurrent pneomopathies 3/10, xerophtalmy 3/10]. Our series deals with 10 cases of HED, consisting in Chris-Siemens Taos-nine syndrome. It highlights the delayed diagnosis of Ibis disease [mean: 14 years] with a diagnosis made at an adult age in four patients. Our study confirm the X-linked heredity [9/10] with a possible autosomal transmission [one female-case]. HED is rarely life-threatening, but early diagnosis allows a better quality of life to patients and genetic counselling to parents. Our series illustrates the rarity of RED which is also probably due to its underestimation by clinicians

[Epidemiologic study of cutaneous cancers in aged persons]

The aging of the population in the developed world is an accepted fact. Consequently, the number of aged patients with cutaneous cancer will increase in coming years. The objective was to determine the nature of skin cancer occurring in elderly patients. We retrospectively assessed all patients of 65 years of age and over, with histologically documented skin cancer, in the department of Dermatology of Charles Nicolle hospital of Tunis between 2000 and 2005. We studied sex, age and the type of tumours. 246patients aged 65 years and over and presenting cutaneous cancers were collected. They represented 57.34% of all cutaneous cancers. The average age was 73 years with a majority [91.46%] between 70 and 85 years. Sex-ratio M/F was 1, 82. Basal cell carcinoma [BCC] represented 68.2% of all cutaneous cancers in aged persons and 53.84% of all BCC independently of age. Squamous cell carcinoma [SCC] represented 23.5% of all cutaneous cancers in aged persons and 67.44% of all SCC independently of age. Eight cases [3.25%] of melanomas in aged persons were collected, representing 61.53% of the 13 cases of melanoma during these 5 previous years, independently of age. More than half of the collected cutaneous cancers during these 5 previous years have occurred in aged persons with a majority between the ages of 70 and 85 years. This is explained by a higher life expectancy in Tunisia. Through this study we conclude that the geriatric patient is at a high risk of developing cutaneous neoplasms especially carcinomas

[Hereditary ichthyosis in Tunisia: epidemiological study of 60 cases]

Ichthyosis are a group of inherited keratinizing disorders. The cutaneous abnormalities may be isolated or associated with extra-cutaneous symptoms. To report the epidemiological and clinical profiles of patients with these genodermatoses from a hospital Tunisian study. A retrospective study of all cases of ichihyosis referred during a period of 5 years to the department of dermatology of Charles Nicole's hospital of Tunis. Sixty cases of hereditary ichihyosis were seen. The sex-ratio was of 0.5. Parental consanguinity was noted in 36 patients [60%]. Seventeen patients [25.7%] had a positive familial history of ichthyosis. The clinical form of ichthyosis was determined in 52 cases. The nonbullous ichthyosiform erythroderma was observed in 25 patients [41.6%]. Sixty patients presented an ichihyosis vulgaris [26.6%]. The other forms of ichthyosis were rarely observed: 4 cases of X-linked recessive ichthyosis, 2 cases of lamellar ichthyosis and 2 cases of bullous iclithyosiform erythroderma. Two patients were born with collodion-like membranes. Two cases presented a complex syndrome. The NBIE, commonly considered as a rare form of ichthyosis, was the most frequently form seen in our study [41.6%], probably because of the high frequency of consanguineous marriages in Tunisia. The IV represents the most frequent form reported in the litterature and was observed in 25% of our patients. The classification of some ichthyosis associated with other extra-cutaneous abnormalities [found in 2 of our patients] remains difficult

Multiple autoimmune syndrome in a patient with pemphigus vulgaris a new combination

The occurrence in the same patient of three or more autoimmune diseases defines multiple autoimmune syndrome [MAS]. Various autoimmune dermatoses, especially pemphigus, have been described in association with systemic autoimmune diseases as component of MAS. The aim of our study is to describe MAS with a new combination in a 55-year-old woman having a pemphigus vulgaris, associated with three other autoimmune diseases. A 55-year-old woman presented with multiple oral erosions of one year and blistering over the trunk and limbs of 15 days. Diagnosis of pemphigus vulgaris was confirmed by histology and direct immunofluorescence on cutaneous biopsy. The patient was started on oral prednisone of 1.25 mg/Kg/day with which the lesions subsided gradually. On day 32 of prednisone, she developed jaundice and purpura. Biology showed an exacerbation of a previous anaemia with thrombocytopenia and elevation of serum aminotransferase, consisting in the diagnosis of autoimmune haemolytic anaemia, autoimmune thrombocytopenia and autoimmune hepatitis. After 40 days of oral steroids [1.25mg/Kg/day], cutaneous lesions, purpura and jaundice had completely resolved. Oral steroids were progressively diminished. The patient was seen after a two-year follow-up period without any skin lesions. Anaemia had improved and there was normal serum aminotransferase. Our patient presented a combination of four autoimmune diseases, i.e. pemphigus, autoimmune haemolytic anaemia, autoimmune thrombocytopenia and autoimmune hepatitis consisting in the diagnosis of type II multiple autoimmune syndrome

[Arteriovenous malformations: study about 54 patients]

Background: Arteriovenous malformation [AVM] are vascular anomalies that may threaten functional and vital prognosis

The aim of this study was to assess the management of Arteriovenous malformation

Methods: it is retrospective study about 54 patients, having AVM, collected over a 7-year period

Results: Sex ratio M/F was 1.25; average age at the diagnosis was 25.4 years. Puberty, pregnancy and trauma, were the most important favoring factors for the development of AVM. Location was the head in 75% of the cases. Doppler ultrasound was the most indicated examination to confirm the diagnosis

In over than 70% of our patients, therapeutic abstention and follow-up were indicated. In the other cases, embolixation, with or without surgery was the treatment. A patient died of massive haemorrhage

Treatment is necessary in complicated AVM. Follow-up [if abstention or after treatment] is mainly based on clinical and Doppler data

Conclusions: AVM are anomalies that may be serious and their management must be multidisciplinary to allow better therapeutic indications